title: Cutting edge issues in the Churg-Strauss syndrome.
abstract: Churg-Strauss syndrome (CSS) is a rare systemic small-vessel vasculitis that develops in the background of bronchial asthma, which is characterized by eosinophilia and eosinophilic infiltration of various tissues. It belongs to the group of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides. The triggering factors and pathogenesis of CSS are still unknown. The possible role of eotaxin-3 and CCR4-related chemokines in selective recruitment of eosinophils to the target tissues in CSS has been recently suggested, but the role of eosinophilic inflammation in the development of vasculitic lesions is not completely understood. From the clinical view, two distinct phenotypes of the disease are slowly emerging depending on the ANCA-positivity status. Glucocorticoids are still the mainstay of treatment; however, data are accumulating regarding the beneficial role of novel immunosuppressants and biologic compounds, especially in patients with poorer prognosis.
authors.affiliation: Department of Medicine, Jagiellonian University Medical College, Skawińska 8, 31 - 066, Kraków, Poland. wszczeklik@gmail.com
authors.forename: Wojciech
authors.initials: W
authors.lastname: Szczeklik
authors.forename: Bogdan
authors.initials: B
authors.lastname: Jakieła
authors.forename: Dariusz
authors.initials: D
authors.lastname: Adamek
authors.forename: Jacek
authors.initials: J
authors.lastname: Musiał
mesh_terms: D002318:Cardiovascular Diseases / Q000150:complications; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D009422:Nervous System Diseases / Q000150:complications; D010641:Phenotype; D011379:Prognosis; D012140:Respiratory Tract Diseases / Q000150:complications; D016896:Treatment Outcome